Heterogeneity of determining disease severity, clinical course and outcomes in systemic sclerosis-associated interstitial lung disease: a systematic literature review

Objective: The course of systemic sclerosis-associated interstitial lung disease (SSc-ILD) is highly variable and different from continuously progressive idiopathic pulmonary fibrosis (IPF). Most proposed definitions of progressive pulmonary fibrosis or SSc-ILD severity are based on the research data from patients with IPF and are not validated for patients with SSc-ILD. Our study aimed to gather the current evidence for severity, progression and outcomes of SSc-ILD. Methods: A systematic literature review to search for definitions of severity, progression and outcomes recorded for SSc-ILD was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines in Medline, Embase, Web of Science and Cochrane Library up to 1 August 2023. Results: A total of 9054 papers were reviewed and 342 were finally included. The most frequent tools used for the definition of SSc-ILD progression and severity were combined changes of carbon monoxide diffusing capacity (DLCO) and forced vital capacity (FVC), isolated FVC or DLCO changes, high-resolution CT (HRCT) extension and composite algorithms including pulmonary function test, clinical signs and HRCT data. Mortality was the most frequently reported long-term event, both from all causes or ILD related. Conclusions: The studies presenting definitions of SSc-ILD 'progression', 'severity' and 'outcome' show a large heterogeneity. These results emphasise the need for developing a standardised, consensus definition of severe SSc-ILD, to link a disease specific definition of progression as a surrogate outcome for clinical trials and clinical practice

True aneurysm of the proximal occipital artery. Case report

INTRODUCTION: True aneurysms of the proximal occipital artery are rare, may cause neurological symptoms due to compression of the hypoglossal nerve and their resection may be technically demanding. PRESENTATION OF CASE: The case of an aneurysm of the proximal occipital artery causing discomfort and tongue deviation by compression on the hypoglossal nerve is reported. Postoperative course after resection was followed by complete regression of symptoms. CONCLUSION: Surgical resection, as standard treatment of aneurysms of the occipital artery, with the eventual technical adjunct of intubation by the nose is effective in durably relieving symptoms and preventing aneurysm-related complicatio